Paediatric pancreatic tumours are rare and mostly malignant. Because they present with vague clinical features, it is important to recognise them and make correct pathological diagnoses using radiological suggestions and histopathology. A one-year-old male child presented to the surgical outpatient department with complaints of abdominal pain for 4 months. The ultrasonography showed a lesion in the left suprarenal region. An ultrasound-guided trucut biopsy showed tumour cells with predominant glandular (acinar) and focal solid (squamoid) patterns with areas of necrosis and haemorrhage. A diagnosis of epithelial cell tumour was given, and IHC was advised. The PET CT scan showed an avidly heterogeneously enhanced large, well-defined soft tissue density lesion involving the left upper abdomen, measuring 5.6x5.6x4.9 cm. The lesion showed an internal zone of necrosis, haemorrhage, and calcification. However, no radiological possibility of a malignant mass lesion of the pancreas was suggested. On immunohistochemistry, the tumour cells were positive for AE1, CK7, beta-catenin, and AFP, along with Ki67, in 70% of tumour nuclei. Based on the morphology, immunohistochemistry, and clinicopathological findings, a diagnosis of pancreatoblastoma was given, and serum AFP levels were suggested. The mass was completely excised, and typical morphological features of the tumor were identified. The definite diagnosis may be challenging, especially when dealing with limited tumour tissue in biopsies. Integration of clinical, radiological, and histopathological findings is essential in pancreatoblastoma diagnosis. In cases of unusual presentation with vague radiologic findings, thorough knowledge of the histopathological features along with a precise IHC panel may help arrive at the diagnosis. Keywords: Pancreatoblastoma, Clinicoradiology, Histopathology, IHC, Survival
Read full abstract